Monitoring of Hemodynamics With Right Heart Catheterization in Children With Pulmonary Arterial Hypertension. 2023

Julien Grynblat, and Sophie-Guiti Malekzadeh-Milani, and Mathilde Meot, and Frédéric Perros, and Isabelle Szezepanski, and Stéphane Morisset, and Caroline Ovaert, and Caroline Bonnet, and Pascale Maragnes, and Julien Ranchoup, and Marc Humbert, and I David Montani, and Marilyne Levy, and Damien Bonnet
M3C-Necker, Hôpital Necker-Enfants malades, AP-HP Université de Paris Cité, Cardiologie Congénitale et Pédiatrique Paris France.

Background Right heart catheterization (RHC) is a high-risk procedure in children with pulmonary arterial hypertension without clear guidelines for the indications and targets of invasive reassessment. Our objectives are to define the aims of repeated RHC and evaluate the correlation between noninvasive criteria and hemodynamic parameters. Methods and Results Clinical and hemodynamic characteristics from 71 incident treatment-naïve children (median age 6.2 years) with pulmonary arterial hypertension who had a baseline and reevaluation RHC were analyzed. Correlations between noninvasive predictors and hemodynamic parameters were tested. Adverse outcomes were defined as death, lung transplantation, or Potts shunt. At baseline, pulmonary vascular resistance index (hazard ratio [HR] 1.07 per 1 WU·m2 increase [95% CI, 1.02-1.12], P=0.002), stroke volume index (HR 0.95 per 1 L·min-1·m-2 increase [95% CI, 0.91-0.99], P=0.012), pulmonary artery compliance index (HR 0.16 per 1 mL·mm Hg-1·m-2 increase [95% CI, 0.051-0.52], P=0.002), and right atrial pressure (HR, 1.31 per 1 mm Hg increase [95% CI, 1.01-1.71], P=0.043) were associated with adverse outcomes. Pulmonary vascular resistance index, pulmonary artery compliance index, and right atrial pressure were still associated with a worse outcome at second RHC. Noninvasive criteria accurately predicted hemodynamic evolution; however, 70% of the patients who had improved based on noninvasive criteria still presented at least 1 "at risk" hemodynamics at second RHC. Conclusions Pulmonary vascular resistance index, pulmonary artery compliance index, and right atrial pressure are solid predictors of adverse outcomes in pediatric pulmonary arterial hypertension and potential therapeutic targets. Noninvasive criteria accurately predict the evolution of hemodynamic parameters, but insufficiently. Repeated RHC are helpful to identify children with persistent higher risk after treatment introduction.

UI MeSH Term Description Entries
D011651 Pulmonary Artery The short wide vessel arising from the conus arteriosus of the right ventricle and conveying unaerated blood to the lungs. Arteries, Pulmonary,Artery, Pulmonary,Pulmonary Arteries
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D006328 Cardiac Catheterization Procedures in which placement of CARDIAC CATHETERS is performed for therapeutic or diagnostic procedures. Catheterization, Cardiac,Catheterization, Heart,Heart Catheterization,Cardiac Catheterizations,Catheterizations, Cardiac,Catheterizations, Heart,Heart Catheterizations
D006439 Hemodynamics The movement and the forces involved in the movement of the blood through the CARDIOVASCULAR SYSTEM. Hemodynamic
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000081029 Pulmonary Arterial Hypertension A progressive rare pulmonary disease characterized by high blood pressure in the PULMONARY ARTERY. Arterial Hypertension, Pulmonary,Hypertension, Pulmonary Arterial
D065627 Familial Primary Pulmonary Hypertension Familial or idiopathic hypertension in the PULMONARY CIRCULATION which is not secondary to other disease. Heritable Pulmonary Arterial Hypertension,Idiopathic Pulmonary Arterial Hypertension,Idiopathic Pulmonary Hypertension,Pph1 With Hht,Primary Pulmonary Hypertension,Pulmonary Hypertension, Primary, 1,Pulmonary Hypertension, Primary, 1, With Hereditary Hemorrhagic Telangiectasia,Pulmonary Hypertension, Primary, Dexfenfluramine-Associated,Pulmonary Hypertension, Primary, Fenfluramine-Associated,Hypertension, Idiopathic Pulmonary,Hypertension, Primary Pulmonary,Pulmonary Hypertension, Idiopathic,Pulmonary Hypertension, Primary

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